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Improved Survival in Left Liver-Up Congenital Diaphragmatic Hernia by Early Repair Before Extracorporeal Membrane
David W Kays1, James L Talbert2, Saleem Islam2
1Department of Surgery, University of Florida, Shands Children's Hospital, Gainesville, FL; Department of Surgery, Johns Hopkins University, St Petersburg, FL.
Insights
Early surgical repair of congenital diaphragmatic hernia (CDH) before extracorporeal membrane oxygenation (ECMO) significantly improves survival in infants with left liver-up CDH. Predictive models can identify high-risk infants for timely intervention.
Area of Science:
- Pediatric Surgery
- Neonatal Intensive Care
- Congenital Malformations
Background:
- Delayed repair of congenital diaphragmatic hernia (CDH) is standard but can complicate treatment for severe cases requiring ECMO.
- Left liver-up CDH has a low survival rate (45%), with deaths often linked to late or no repair after ECMO initiation.
- Early prediction of ECMO risk is crucial for identifying optimal candidates for pre-ECMO surgical repair.
Purpose of the Study:
- To predict ECMO risk in infants with left liver-up CDH.
- To evaluate the impact of surgical timing on survival in these high-risk infants.
- To develop a predictive model for ECMO need based on early clinical markers.
Main Methods:
- Retrospective review of 298 CDH patients, focusing on 87 inborn left liver-up cases without lethal anomalies.
- Multivariate analysis of anatomic and physiologic severity markers to determine associations with ECMO requirement.
- Development and validation of multivariate models to predict ECMO risk using early life data.
Main Results:
- 69% (60/87) of eligible infants required ECMO.
- Survival was significantly higher for infants repaired within 60 hours and before ECMO (95%; 20/21) compared to those repaired later after ECMO initiation (65%; 13/20).
- Lung-to-head ratio, Apgar scores, CDH Study Group survival prediction, and blood gas levels at 1 hour strongly correlated with ECMO risk; predictive models achieved AUCs of 0.91.
Conclusions:
- Early surgical repair of left liver-up CDH before ECMO significantly improves survival outcomes.
- Accurate multivariate models can predict ECMO risk at 1 hour of life.
- Stratifying surgical timing based on early risk assessment can maximize survival potential in CDH patients.
Background:
Delayed repair of congenital diaphragmatic hernia (CDH) for days or longer has become standard, allowing improved stabilization for many, but potentially complicating treatment in severely affected infants who require extracorporeal membrane oxygenation (ECMO) and arrive unrepaired. Survival in left liver-up CDH, the most severe anatomic subset, averages 45% in published studies, with deaths often occurring in patients who failed to improve on ECMO and are repaired late, or not at all. Reliable early prediction of ECMO risk in these patients could identify the best candidates for repair before ECMO. We sought to predict ECMO risk in left liver-up CDH, and to further evaluate survival stratified by surgical timing in these patients.
Study Design:
We reviewed 298 single-center, consecutive CDH patients, focusing on 87 inborn left liver-up patients without associated lethal anomalies. Multivariate analysis using anatomic and physiologic markers of severity was performed to define associations with need for ECMO.
Results:
Sixty of 87 ECMO-eligible inborn left liver-up CDH patients required ECMO (69%). Of these, 20 of 21 (95%) repaired in the first 60 hours and before ECMO survived; whereas 13 of 20 (65%) who had repair delayed and arrived to ECMO unrepaired survived (p = 0.018). Lung-to-head ratio, Apgar scores, Congenital Diaphragmatic Hernia Study Group-predicted survival, pH, PCO2, and PO2 at 1 hour of life all correlated strongly with risk for ECMO. Accurate multivariate models to predict ECMO (area under the receiver operating characteristic curve [AUC] 0.91 and 0.91) were successfully developed.
Conclusions:
Early repair of left liver-up CDH before ECMO results in improved survival. Multivariate models can accurately assess risk for ECMO at 1 hour of life, permitting stratification of CDH surgical timing to maximize survival potential while minimizing risk.
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